This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. This is a pilot study involving 11 sub-sites to test the efficacy with NIPPV and nutritional management in patients with ALS. Six sites will be involved with early NIPPV intervention, and five sites will be involved in nutritional management intervention, and each site will recruit 20 patients. The primary goals of the NIPPV study arm are to develop and validate strategies to improve tolerability of NIPPV, to identify factors that influence acceptance of this treatment modality, and to evaluate measures of early respiratory failure. The primary goals of the early nutrition management arm are to develop and validate methods to determine energy balance in ALS patients that will be applicable to a multicenter Phase III study of nutrition and NIPPV.